Surgical creation of lymphocutaneous fistulas for the management of infants with central lymphatic obstruction

Purpose Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca. Methods We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status. Results We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68–265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1–8.1] kg vs. 6.1 [IQR 4.9–7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25–31] cmH 2 O vs. 22 [IQR 22–24] cmH 2 O, P = 0.005; 36 [IQR36–38] cmH 2 O vs. 33 [IQR 33–35] cmH 2 O, P = 0.002; 36 [IQR 34–47] cmH 2 O vs. 28 [28–31] cmH 2 O, P = 0.002). LCFs remained patent for 29d (IQR 16–49d). LCFs contracted over time, and 6/11 (54.5%) were eventually revised. There were no complications. Two patients died from overwhelming disease, one died from unrelated causes, and one remains alive 29 months after their initial LCF. Conclusion LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling. Level of evidence IV.